CASE REPORT http://doi.org/10.29289/25945394202020200062

Pseudoangiomatous stromal of the presenting as gigantomastia: case report

Juliana Pontes Farias1* , Humberto Espínola Guedes Neto1 , Ariano Brilhante Pegado Suassuna1 , Rafael Chaves Claudino de Queiroga1 , Arthur Antonino da Silva Nunes1 , Adriano de Lima Quirino1 , Thaíse Lopes Medeiros1

ABSTRACT

Pseudoangiomatous stromal hyperplasia (PASH) of the breast is a benign condition generally seen as an incidental finding of biopsies for other causes. In some rare cases, it can evolve with expressive growth of breast tissue, leading to large . The present study presents a case report of a patient who started with breast hypertrophy during lactation, evolving with gigantomastia, and arrived at the office 3 years after gestation with breasts of 6.2 and 4.3 kg and growth of accessory breast and axillary lymph nodes. The patient was emaciated, with bodily pain and psychological distress. Computed tomography was performed with the finding of diffuse retroglandular nodules and axillary and mediastinal lymph node enlargement, without being able to rule out the lymphoproliferative process. Then, core biopsies were made in several places on the breast whose pathological examination revealed PASH samples. Bilateral was performed as a surgical treatment, with resection of the left accessory breast and left axillary lymph node, and breast reconstruction, with placement of silicone prostheses and graft of the -papillary complex, together with a plastic surgeon. She evolved in the postoperative period with good healing of the surgical wound, weight gain, necrosis of the areola-papillary complex and with severe psychiatric disorder, which was treated appropriately by psychiatrists, with remission of symptoms. This case report presents a rare evolution of PASH with gigantomastia, bringing intense physical and psychological distress to the patient, whose treatment chosen was total surgical resection of the breast tissue and aesthetic reconstruction. KEYWORDS: breast ; breast implantation; lactation; mastectomy; pathology.

INTRODUCTION CASE REPORT Pseudoangiomatous stromal hyperplasia (PASH) is a rare benign A 27-year-old woman was referred to the outpatient mastology condition of the breasts first described in 19861, most commonly service at the Hospital Universitário Lauro Wanderley (HULW), in found in women in pre- and perimenopause2 with findings on João Pessoa, Brazil, with the complaint of bilateral gigantomas- imaging tests not specific to this condition, requiring a correlation tia accompanied by weight loss. She was seen for the first time with the anatomopathological aspect for diagnostic confirma- in August 2019, reporting breast enlargement that started dur- tion3. PASH can coexist with other breast lesions or even mimic ing (three years ago) and worsened during lactation. fibroadenoma and it can progress, in spite of its being a benign After the first consultation, the patient did not attend the disease4. The diagnosis of this condition usually occurs inciden- return visit and only in February 2020 did she return to the clinic tally during investigation with anatomopathological study of for further investigation. The breasts had almost doubled in size other benign or malignant diseases of the breast5. Treatment var- compared to the first consultation, with engorged vessels and ies depending on the presentation. areas with necrosis and suppuration (Figure 1). Diagnostic hypoth- The objective of this study was to report a case with atypical eses were raised for PASH and breast lymphoma, both rare condi- evolution and surgical treatment with bilateral mastectomy and tions. Malignant was considered due to rapid growth, immediate reconstruction, since the patient presented a rare and the presence of axillary lymph node enlargement and signifi- diffuse PASH condition beginning in pregnancy with rapid and cant weight loss. Complementary investigation did not include bilateral breast enlargement. mammography due to the patient’s age and, mainly, due to the

1Hospital Universitário João Pessoa, Universidade Federal da Paraíba – João Pessoa (PB), Brazil. *Corresponding author: [email protected] Conflict of interests: nothing to declare. Received on: 10/04/2020. Accepted on: 11/09/2020.

Mastology 2020;30:e20200062 1 Farias JP, Guedes Neto HE, Suassuna ABP, Queiroga RCC, Nunes AAS, Quirino AL, Medeiros TL

impossibility of performing it in view of the technical limita- tions given the expressive size of the breasts. On the day of the consultation, a computed tomography (CT) scan of the chest was performed, with and without iodinated venous contrast, revealing an important volumetric increase in the breasts due to the multiple confluent nodular formations, predominantly retroglandular, which exhibited slight impregnation by means of contrast, of etiology indeterminate to the method, and it was not possible to discard a lymphoproliferative process. The pres- ence of multiple lymph node enlargement in axillary chains and in the internal chest wall bilaterally also stands out (Figures 2A and 2B). A laboratory screening with general exams and serology was requested to rule out associated conditions and to obtain the necessary exams for surgery. Fragments of the left breast were also collected by means Figure 1. (A) Front view of the breasts; (B) right breast in lateral of a core-biopsy for histopathological analysis. The result was view, with suppurative ; (C) left breast in lateral view and released on February 10th, 2020, revealing breast tissue with accessory breast (photographs taken in February 2020). pseudoangiomatous stromal hyperplasia (morphological aspects

Figure 2. (A) Chest computed tomography shows a volumetric increase in the breasts due to multiple confluent nodular formations, predominantly retroglandular; (B) chest computed tomography highlighting the presence of multiple lymph node enlargements in axillary chains and the presence of the accessory breast on the left; (C) surgical parts of the direct and left breast (top to bottom); (D) anatomopathological showing stromal pseudoangiomatous hyperplasia, nodular and diffuse forms; (E) lymph nodes with reactive lymphoid hyperplasia; (F) absence of signs of malignancies.

2 Mastology 2020;30:e20200062 Pseudoangiomatous stromal hyperplasia of the breast presenting as gigantomastia: case report

consistent with the diffuse form of PASH) and absence of signs hyperplasia; skin without particularities; absence of signs of malignancy. of malignancy; On March 12th, 2020, the patient underwent bilateral total • Axillary lymph nodes on the left (dimensions = 2.5 × 2 × mastectomy and immediate breast reconstruction by the ser- 0.8 cm): lymph nodes with reactive lymphoid hyperplasia; vices of mastology and , respectively, at HULW. absence of signs of malignancy. Both breasts, the left accessory breast, and the left axillary lymph nodes were resected (Figures 2C and 3A). The areola-papillary In the first postoperative visit, the patient returned without complex was preserved and used as a graft in the reconstruc- complaints. On physical examination, significant edema was tion of the breasts, and silicone prostheses were implanted in a observed in the inframammary folds (Figure 3C). On the sec- submuscular position (Figure 3B). ond return, she presented remission of these findings and com- Surgical specimens were sent for histopathological study at plained of delusions, being referred to the psychiatric service, the Pathological Anatomy Laboratory (Laboratório de Anatomia where drug treatment was started. In the third consultation, Patológica – LAP) of the same hospital (Figures 2D, 2E, and 2F). she showed improvements in psychiatric symptoms and the The results, released on March 26th, 2020, were: removal of the surgical stitches was performed. Partial necrosis • Right breast (weight = 6,255 g; dimensions = 38 × 32 × 10 cm): of the areola-papillary complex grafts and progressive weight pseudoangiomatous stromal hyperplasia, nodular and gain, around 15 kg, were observed in relation to the beginning diffuse forms; skin without particularities; absence of signs of the follow-up (Figure 3D). The possibility of a corrective sur- of malignancy; gical procedure was offered, however, the patient expressed no • Left breast (weight = 4,295 g; dimensions: 29 × 27 × 12 cm): interest in performing a new surgery. pseudoangiomatous stromal hyperplasia, nodular and The study was carried out according to the ethical princi- diffuse; skin without particularities; absence of signs of ples of studies in human beings according to the Declaration of malignancy; Helsinki, with the approval of the Research Ethics Committee • Left accessory breast (dimensions = 11 × 8 × 5 cm): of Hospital Universitário Lauro Wanderley, with CAAE number ectopic breast tissue with pseudoangiomatous stromal 36548520.2.0000.5183.

Figure 3. (A) marking and skin flaps after mastectomy; (B) frontal and lateral view of the immediate postoperative period of breast reconstruction after total mastectomy with resection of the left accessory breast; (C) postoperative period of April 2020, showing signs of partial necrosis of the areola-papillary complex grafts; (D) Late postoperative period of September 2020, showing good healing of the surgical wound, in addition to the patient’s weight gain.

Mastology 2020;30:e20200062 3 Farias JP, Guedes Neto HE, Suassuna ABP, Queiroga RCC, Nunes AAS, Quirino AL, Medeiros TL

DISCUSSION presence of weight loss and rapid growth, which was discarded PASH is a benign stromal proliferation that can affect different by breast biopsy. age groups, with a mean age ranging between 37 and 51 years6, In order to confirm the diagnosis, core-biopsy is sufficient with up to 75% of pre-menopausal patients7. The patient in this and necessary in cases of abnormal findings on imaging or case was affected at a younger age than the average. physical examination4. In the case of the patient under study, The precise etiology of PASH is unknown, but are the tomographic finding did not rule out the presence of lym- attributed a role in its development2, considering that almost all phoproliferative disease in the breasts, indicating the need for affected postmenopausal women were using hormone8. investigative supplementation with a core- biopsy, before sched- There are typically two clinical types of PASH — nodular and uling surgical excision. diffuse. The nodular type is characterized by a unilateral, circum- The management of PASH depends on its presentation8. scribed, slow-growing mass and is usually an incidental finding Some studies recommend mastectomy in diffuse type cases3,4,8 that mimics fibroadenomas. The diffuse type is more rare, with and others report9,15 that immediate breast reconstruction was about 20 cases reported in the literature9. The involvement of the also performed. The surgical approach of the case is well indi- areola-papillary complex and the axillary accessory breast are cated, in view of the diffuse and rapid growth and its interfer- also atypical findings10. In this respect, the case has unusual char- ence in the quality of life. acteristics, such as bilateral involvement, diffuse growth, impos- sibility to delimit the tumor, and the onset of an axillary acces- sory breast. Only two cases of PASH beginning with pregnancy CONCLUSION have been reported9,11, as happened in the present study’s patient. The present case revealed a rare breast condition with atypical A case of axillary lymph node enlargement is described in evolution, due to rapid, diffuse, and bilateral progression. Due to the literature, probably reactional to edema12. The patient in the course of the disease in the patient, a surgical procedure for the present case also had a lymph node with reactive lymphoid bilateral mastectomy and immediate breast reconstruction with hyperplasia. prosthesis implantation was indicated. This experience rein- Ultrasonographic findings are variable, ranging from circum- forces the need for good propaedeutic management of PASH and scribed to indistinct tumors13, more commonly with the presence adequate treatment according to the clinical picture presented, of a hypoechoic and heterogeneous nodule14. A study that evalu- especially due to the lack of specific consensus or protocols for ated ultrasound findings in patients with PASH classified 93.6% the disease. The individualized treatment of each patient is cur- of the cases as BI-RADS 4, due to the fact that these exams pres- rently the best option, considering clinical evolution, aesthetic ent images with non-circumscribed margins14. One year before aspects, and results of complementary exams. the appointment, the patient underwent breast ultrasound, in which no nodules or changes had been observed. On mammog- raphy, solid, non-calcified and circumscribed nodules are typi- AUTHORS’ CONTRIBUTIONS cally found, with focal asymmetry3. Most studies do not provide J.P.F.: supervision, methodology, writing — review. a description of this pathology on CT, therefore, this case proves H.E.G.N.: methodology, writing — original draft & editing. to be opportune as it presents this characterization. A.B.P.S.: methodology, writing — original draft & editing. The main differential diagnoses, due to the clinical or histo- R.C.C.Q.: methodology, writing — original draft & editing. logical aspects of PASH, are: fibroadenoma, phyloid tumor, and A.A.S.N.: methodology, writing — original draft & editing. low-grade angiosarcoma2,12,14. In the present case, diagnostic T.L.M.: methodology, writing — review & editing. doubts regarding breast lymphoma were included, due to the A.L.Q.: methodology, review.

REFERENCES

1. Vuitch MF, Rosen PP, Erlandson RA. Pseudoangiomatous Stromal Hyperplasia of the Breast: Multimodality hyperplasia of mammary stroma. Hum Pathol. 1986;17(2):185‑91. Review With Pathologic Correlation. Curr Probl Diagn https://doi.org/10.1016/s0046-8177(86)80292-1 Radiol. 2017;46(2):130-5. http://doi.org/10.1067/j. 2. Smilg P. Pseudoangiomatous stromal hyperplasia: Presentation cpradiol.2016.01.005 and management – a clinical perspective. SA J Radiol. 4. Yoon KH, Koo B, Lee KB, Lee H, Lee J, Kim JY, et al. Optimal 2018;22(2):1366. https://dx.doi.org/10.4102%2Fsajr.v22i2.1366 treatment of pseudoangiomatous stromal hyperplasia 3. Raj SD, Sahani VG, Adrada BE, Scoggins ME, Albarracin of the breast. Asian J Surg. 2020;43(7):735-41. https://doi. CT, Woodtichartpreecha P, et al. Pseudoangiomatous org/10.1016/j.asjsur.2019.09.008

4 Mastology 2020;30:e20200062 Pseudoangiomatous stromal hyperplasia of the breast presenting as gigantomastia: case report

5. Powell CM, Cranor ML, Rosen PP. Pseudoangiomatous Stromal 11. Prichard RS, O’Neill CJ, O’Hara JL, Atmore BB, Hassall M. Hyperplasia (PASH). Am J Surg Pathol. 1995;19(3):270‑7. https:// Pseudoangiomatous stromal hyperplasia of the breast: doi.org/10.1097/00000478-199503000-00004 an unusual pathology necessitating bilateral mastectomy 6. Hoda SA, Brogi E, Koerner FC, Rosen PP. Rosen’s Breast during pregnancy. ANZ J Surg. 2011;81(4):304-5. https://doi. Pathology. 4th ed. Philadelphia: Wolters Kluwer Health/ org/10.1111/j.1445-2197.2011.05688.x Lippincott Williams Wilkins; 2014. 1379 p. 12. Tsuda B, Kumaki N, Ishida R, Sakaeda E, Ishii S, Mizuno M, 7. Bourke AG, Tiang S, Harvey N, McClure R. Pseudoangiomatous et al. Rare finding of bilateral pseudoangiomatous stromal stromal hyperplasia causing massive breast enlargement. BMJ hyperplasia of the breast: A case report. Tokai J Exp Clin Med. Case Rep. 2015;2015:bcr2014204343. https://doi.org/10.1136/ 2019;44(4):73-9. bcr-2014-204343 13. Polger MR, Denison CM, Lester S, Meyer JE. Pseudoangiomatous 8. Nascimento TC, Djahjah MC, Carneiro AHPC, Oliveira AC stromal hyperplasia: mammographic and sonographic de, Marchiori E. Pseudoangiomatous stromal hyperplasia appearances. Am J Roentgenol. 1996;166(2):349-52. https://doi. presenting as a tumor. Radiol . 2019;52(2):128-9. https:// org/10.2214/ajr.166.2.8553945 doi.org/10.1590/0100-3984.2017.0135 14. Amorim HLE, Torres AF, Macedo Filho MAA, Paz AR, 9. Krawczyk N, Fehm T, Ruckhäberle E, Mohrmann S, Riemer Pereira GCG. Análise dos achados ecográficos em nódulos J, Braunstein S, et al. Bilateral Diffuse Pseudoangiomatous de hiperplasia pseudoangiomatosa do estroma mamário Stromal Hyperplasia (PASH) Causing Gigantomastia in a diagnosticados por core biópsia. Rev Bras Mastol. 33-Year-Old Pregnant Woman: Case Report. Breast Care. 2011;21(2):66‑9. 2016;11(5):356-8. https://doi.org/10.1159/000450867 15. Soares AB, Queiroga RC, Soares BR, Maximiano AMC, Cardoso 10. Virk RK, Khan A. Pseudoangiomatous Stromal Hyperplasia: IF, Leal PR. Reconstrução mamária imediata após ressecção An Overview. Arch Pathol Lab Med. 2010;134(7):1070-4. https:// de hiperplasia estromal pseudoangiomatosa em adolescente doi.org/10.1043/2008-0686-RS.1 de 13 anos. Rev Bras Mastol. 2011;21(3):127-30.

© 2020 Brazilian Society of Mastology This is an open access article distributed under the terms of the Creative Commons license.

Mastology 2020;30:e20200062 5